Results of a hybrid approach for high risk term newborn patients with interrupted aortic arch (IAA) with left ventricular outflow tract obstruction.

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated. Nine cases underwent hybrid approach during the study period. The mean age and weight at interventions were 7 days (3-16 days) and 3280 g (2700-4300 g). Six of the patients were diagnosed with type B IAA, 2 with type A, and one with type C. LVOTO was present in 7 patients. The success rate for the procedures was 100%. No patients died during the procedure or within the first 5 days after the procedure or from reasons related to the procedure. The median length of the hospital stay after stent placement was 28 days (22-35 days) for discharged patients. Three patients died in interstage period, and 6 patients underwent total corrective surgery after a median of 7 months (4-10 months). The average LVOT diameter was increased from 3.1 mm to 4.8 mm before total repair surgery. The hybrid approach should be kept in mind for treating high risk newborns with IAA with LVOTO and high-risk newborns who are not suitable for single stage total corrective surgery.

Evaluation of perfusion index and left ventricular output changes in low cardiac output syndrome after arterial switch operation.

INTRODUCTION: Transposition of great arteries is one of newborns' most common cyanotic CHDs, and its treatment is arterial switch operation in the first days of life. Low cardiac output syndrome may develop in the early postoperative period. In this study, we evaluated perfusion index and left ventricular output blood flow changes in patients who underwent arterial switch operation and developed low cardiac output syndrome. METHODS: This study was conducted prospectively in newborns with transposition of great arteries who underwent arterial switch operation between 1st August 2020 and 1st August 2022. Low cardiac output syndrome score and left ventricular output were investigated. Initially, 6th, 12th, 18th, and 24th hour perfusion index and left ventricular output values of patients with and without low cardiac output syndrome were recorded. The results were evaluated statistically. RESULTS: A total of 60 patients were included in the study. Sex distribution was equal. The median age at the time of surgery was 5 days (interquartile range 3-7 days), and the median weight was 3.1 kg (interquartile range 2.9-3. 4). Low cardiac output syndrome was detected in 30% (n = 18) of cases. The median perfusion index of patients who developed low cardiac output syndrome was significantly lower at the 12th, 18th, and 24th hours (p < 0.05) (0.99 versus 1.25, 0.86 versus 1.21, and 0.96 versus 1.33, respectively). Similarly, the median left ventricular output of patients who developed low cardiac output syndrome was significantly lower at 12th, 18th, and 24th hours (p < 0.05) (95 versus 110 ml/kg/min, 89 versus 109 ml/kg/min, and 92 versus 112 ml/kg/min, respectively). There was a significant correlation between perfusion index values and left ventricular output at all measurements (r > 0.500, p < 0.05). CONCLUSION: Perfusion index and left ventricular output measurements decreased in newborns who developed low cardiac output syndrome after arterial switch operation, especially at 12th and 18th hours. Serial perfusion index and left ventricular output measurements can be instructive in predicting low cardiac output syndrome development.

Delayed diagnosis of homocystinuria presenting with coronavirus disease 2019 in a 17-year-old boy.

Homocystinuria is a treatable autosomal recessive inherited disorder. This condition may cause life-threatening complications such as thromboembolic events. Coronavirus disease 2019 (COVID-19) is associated with an increased risk of venous thromboembolic events. Here, we report a case of late diagnosis of homocystinuria presenting with deep venous thrombosis and COVID-19. This study highlights a sustained high index of suspicion for homocystinuria to prevent severe thromboembolic complications.

Delayed diagnosis of homocystinuria presenting with coronavirus disease 2019 in a 17-year-old boy

ABSTRACT Homocystinuria is a treatable autosomal recessive inherited disorder. This condition may cause life-threatening complications such as thromboembolic events. Coronavirus disease 2019 (COVID-19) is associated with an increased risk of venous thromboembolic events. Here, we report a case of late diagnosis of homocystinuria presenting with deep venous thrombosis and COVID-19. This study highlights a sustained high index of suspicion for homocystinuria to prevent severe thromboembolic complications.

Late Arterial Switch Surgery Under ECMO Support in a Patient with Transposition of the Great Arteries with Intact Ventricular Septum: a Case Report.

A 30-month-old male patient with transposition of the great arteries with intact ventricular septum (TGA/IVS) is presented. Arterial switch operation (ASO) was performed in the light of echocardiographic and angiographic findings. The patient remained under extracorporeal membrane oxygenation support for seven days postoperatively, and his cardiac functions returned to normal at the postoperative 10th day. He was discharged at the postoperative 20th day. The present case, which presents one of the most advanced ages at operation for TGA/IVS among previously reported cases, is used to discuss late ASO in this study.

Late arterial switch surgery under ecmo support in a patient with transposition of the great arteries with intact ventricular septum: a case report

Abstract A 30-month-old male patient with transposition of the great arteries with intact ventricular septum (TGA/IVS) is presented. Arterial switch operation (ASO) was performed in the light of echocardiographic and angiographic findings. The patient remained under extracorporeal membrane oxygenation support for seven days postoperatively, and his cardiac functions returned to normal at the postoperative 10th day. He was discharged at the postoperative 20th day. The present case, which presents one of the most advanced ages at operation for TGA/IVS among previously reported cases, is used to discuss late ASO in this study.

Vacuum-Assisted Closure for Mediastinitis in Pediatric Cardiac Surgery: A Single-Center Experience.

Vacuum-assisted closure (VAC) has been widely used to treat mediastinitis after congenital cardiac surgery, which is associated with a high risk of morbidity and mortality. The aim in this study is to review our 14 cases of mediastinitis treated with VAC therapy after congenital cardiac surgery. We retrospectively reviewed the medical records of 14 congenital heart patients with mediastinitis from January 2012 to March 2017. Patients with fever, wound discharge, sternal dehiscence, a positive wound culture or abscess diagnosed with computed tomography are accepted as mediastinitis. A VAC was applied to all our patients without irrigation or dressing the wound because of sterility concerns. The vacuuming of the wound was either 50 mm Hg or 75 mm Hg according to the sternal intactness. We gradually decreased the pressures and changed the VAC systems once every three days, after wound healing was seen and a negative culture was obtained and VAC was terminated. There were 14 patients (8 male and 6 female) with mediastinitis and all of them were treated using VAC. The mean age of the patients was 6.96 months (ranging from 0.5-26 months). The mean weight was 5.16 kg (2.8-12 kg). Three patients needed extracorporeal membrane oxygenation after the surgery. Mean onset of mediastinitis was 25.3 days. The wound cultures showed methicillin resistant coagulase negative streptococcus and methicillin-sensitive staphylococcus aureus in most cases. Acinetobacter, serratia, pseudomonas, and klebsiella were the other bacterial species seen in cultures. Two patients had mediastinitis symptoms, but their cultures were negative. VAC systems were changed 3.85 times on average. Mean duration of hospital stay was 49.9 days (21-104 days). One patient needed a muscle flap to close the thoracic cavity after mediastinitis. Two patients did not survive. Mediastinitis is a serious postoperative condition in pediatric cardiac surgery patients. Classical wound dressing and irrigation methods are not suitable in mediastinitis for the pediatric age group. Therefore, VAC therapy can be an effective way to successfully treat the situation.

Analysis of congenital heart surgery results: A comparison of four risk scoring systems.

BACKGROUND: This study aims to evaluate the surgical results of our clinic according to presumption systems of Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity score, Aristotle Comprehensive Complexity score, and Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories and to compare the efficiency of these systems in predicting morbidity and mortality. METHODS: In the study, classification and the risk scoring were performed with the four different systems for 1,950 patients (1,038 males, 912 females; mean age 5.5 months; range, 1 day to 18 years) who were administered congenital heart surgery between 1 October 2012 and 31 December 2016. The hospital mortality and morbidity were calculated for each category from the four models. The discriminatory ability of the models was determined by calculating the area under the receiver operating characteristic curve and the receiver operating characteristic curves of the four models were compared. RESULTS: Median weight of the patients was 7.2 kg (range, 1.8-80 kg). Among the patients, 53% were males and 47.5% were younger than one year of age. Of totally 1,950 operations, mortality was observed in 149 (7.6%) and morbidity was observed in 541 (27.7%). Areas under the receiver operating characteristic curve for mortality were 0.803, 0.795, 0.729, and 0.712 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Aristotle Comprehensive Complexity, Risk Adjustment in Congenital Heart Surgery, and Aristotle Basic Complexity scores, respectively. Areas under the receiver operating characteristic curve for morbidity were 0.732, 0.731, 0.730, and 0.685 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Comprehensive Complexity, and Aristotle Basic Complexity scores, respectively. CONCLUSION: Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity, and Aristotle Comprehensive Complexity score systems were effective in predicting the morbidities and mortalities of patients who underwent congenital heart surgery and evaluating the performance of the surgical centers. Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories were on the forefront due to high feasibility and performance. Aristotle Basic Complexity score system had the lowest performance. Combinations of systems will provide the most benefit during evaluation of results.

The use of neonatal extracorporeal life support in pediatric cardiac intensive care unit.

AIM: The aim of the study is to evaluate extracorporeal life support system (ECLS) employed in neonates in pediatric cardiac intensive care unit. MATERIAL AND METHODS: Twenty-five neonates that required ECLS in between November 2010 and November 2015 were evaluated. RESULTS: The median age was 12 days (range 3-28 days) and the median body weight was 3 kg (range 2.5-5 kg). Venoarterial ECLS was performed in all of the cases. Ascendan aorta-right atrial cannulation in 22 patients and neck cannulation in three patients were performed. The reason for ECLS was E-CPR in two patients, inability to wean from cardiopulmonary bypass (CPB) in seven patients, respiratory insufficiency and hypoxia in nine patients, low cardiac output (LCOS) in seven patients. Median duration of ECLS was four days (range 1-15). Hemorrhagic complications developed in 15, renal complications in 13, pulmonary complications in 12, infectious complications in 11, neurologic complications in three and mechanical complications in two of the patients. Weaning was successful in 15 of the patients. Eleven patients were successfully discharged. CONCLUSION: ECLS is an important treatment option that is performed successfully in many centers around the world to maintain life support in patients unresponsive to medical treatment. The utilization of this modality especially in newborns with congenital heart disease should be taken into consideration.

How to approach aortic valve disease in the elderly: a 25-year retrospective study.

OBJECTIVE: In the last decade, the number of elderly patients suffering from aortic valve disease has significantly increased. This study aimed to identify possible factors that could affect surgical and long-term outcomes in the light of a literature review regarding the management of aortic valve disease in the elderly. METHODS: Between January 1990 and December 2012, a total of 114 patients (64 males, 50 females; mean age 76.6 ± 3.6 years; range 70-87 years) with aortic valve replacement (AVR) alone, or combined with coronary artery bypass grafting (CABG) or mitral surgery in our hospital, were retrospectively analysed. RESULTS: In-hospital mortality was seen in 19 patients. The major causes of in-hospital mortality were low-cardiac output syndrome in eight patients (42.1%), respiratory insufficiency or infection in six (31.5%), multi-organ failure in four (21%), and stroke in one patient (5.2%). The main postoperative complications included arrhythmia in 26 patients (22.8%), renal failure in 11 (9.6%), respiratory infection in nine (7.9%), and stroke in three patients (2.6%). The mean length of intensive care unit and hospital stays were 6.4 ± 4.3 and 18 ± 12.8 days, respectively. During follow up, late mortality was seen in 28 patients (29.4%). Possible risk factors for long-term mortality were type of prosthesis, EuroSCORE ≥ 15, postoperative pacemaker implantation, respiratory infection, and haemodialysis. Among 65 long-term survivors, their activity level was good in 53 (81.5%) and poor in two. CONCLUSIONS: Our study results demonstrated that an individually tailored approach including scheduled surgery increases short- and long-term outcomes of AVR in patients aged ≥ 70 years. In addition, shorter cardiopulmonary bypass time may be more beneficial in this high-risk patient population.

Introduction to a Norwood program in an emerging economy: learning curve of a single center.

BACKGROUND: There has been a notable improvement in the outcome of stage 1 palliation for hypoplastic left heart syndrome (HLHS) in recent years. Nevertheless, developing a new Norwood program requires a steep learning curve, especially in emerging economies where rapid population growth brings a high volume of patients but, on the other hand, resources are limited. In this paper we aimed to summarize the initial results of a single center. METHODS: Hospital records of 21 patients were reviewed for all patients having a stage 1 palliation procedure for HLHS between May 2011 and May 2013. There were 13 male (62%) and 8 female (38%) patients. Median age was 14 days (range, 4-74 days) and median weight was 3030 g (2600-3900 g). HLHS was defined as mitral or aortic stenosis or atresia (or both) in the presence of normally related great vessels and a hypoplastic left ventricle. Transthoracic echocardiography was the diagnostic modality used in all patients. All procedures but one were performed using an antegrade selective cerebral perfusion method and moderate hypothermia. Cerebral perfusion was monitored with cerebral oximetry in all patients. Modified ultrafiltration was routinely used in all patients. RESULTS: Overall hospital mortality was 47.6% (n = 10). Mortality rates considerably decreased from the first year to second year (69% and 12.5% respectively). No risk factors were identified for mortality. CONCLUSIONS: Surgical palliation of neonates with hypoplastic left heart syndrome continues to be a challenge. To decrease the overall mortality nationwide and improve outcomes, a referral center with a dedicated team is necessary in emerging economies.